CF is a genetic disease characterized by abnormalities in sodium, chloride, and water transport across epithelial cells, resulting in dehydration and thickening of the mucus layer in affected cells. In the lungs, dehydration of airway secretions results in increased mucus viscocity. The inability of patients with CF to clear this thickened mucus is due, at least in part, to impaired funcioning of the mucociliary escalator. Chronic lung infection results, leading to progressive lung disease... triphosphates such as UTP regulate activ.